title: Comparison of clinical features of arrhythmogenic right ventricular cardiomyopathy in men versus women.
abstract: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heart muscle disease characterized by myocardial necrosis followed by fibrous-fatty replacement. The pathologic process constitutes the basis for ventricular arrhythmias due to re-entrant circuits. Even if this genetic disease is transmitted in the majority of cases with autosomal dominant trait, in all reported series ARVC is prevalent in men. In this study we investigate the impact that gender may have on clinical presentation in a large series of patients with ARVC. A total of 171 consecutive patients (mean 29 +/- 12 years, range 13 to 65) affected by ARVC were examined with family and personal history, 12-lead electrocardiogram (ECG), 24-hour ECG, signal-averaged ECG, and echocardiogram. Moreover, electrophysiological study and ventricular angiography were performed in selected cases. In the 171 subjects, 71% were men and 29% women (p = 0.02). No gender differences were found considering the age at the time of diagnosis and of study enrolment and the prevalence of index cases and family members. The genders differed in prevalence of abnormal ECG (69% vs 52%, p = 0.036) and presence of late potentials (60% vs 40%, p = 0.01). Moreover, men had larger right ventricular dimensions and practiced competitive sports more frequently (26% vs 14%, p <0.001). Nonetheless, gender was not associated with a high incidence of life-threatening ventricular arrhythmias or with a poor outcome. In conclusion, our data show that diagnosis of ARVC is less common in female patients, who present a higher prevalence of mild forms. Nonetheless, the degree of electrical instability does not differ significantly between genders in affected subjects. Even if ARVC remains mainly a male disease, gender does not have a role in patients' outcome. The cause of the under-representation of women is not clear, even if potentially important factors such as sexual hormones and physical activity could play a role.
authors.affiliation: Department of Cardio-thoracic and Vascular Sciences, University of Padua Medical School, Padua, Italy.
authors.forename: Barbara
authors.initials: B
authors.lastname: Bauce
authors.forename: Gianfranco
authors.initials: G
authors.lastname: Frigo
authors.forename: Frank I
authors.initials: FI
authors.lastname: Marcus
authors.forename: Cristina
authors.initials: C
authors.lastname: Basso
authors.forename: Alessandra
authors.initials: A
authors.lastname: Rampazzo
authors.forename: Francesco
authors.initials: F
authors.lastname: Maddalena
authors.forename: Domenico
authors.initials: D
authors.lastname: Corrado
authors.forename: Mikolaj
authors.initials: M
authors.lastname: Winnicki
authors.forename: Luciano
authors.initials: L
authors.lastname: Daliento
authors.forename: Ilaria
authors.initials: I
authors.lastname: Rigato
authors.forename: Alexandros
authors.initials: A
authors.lastname: Steriotis
authors.forename: Elisa
authors.initials: E
authors.lastname: Mazzotti
authors.forename: Gaetano
authors.initials: G
authors.lastname: Thiene
authors.forename: Andrea
authors.initials: A
authors.lastname: Nava
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000175:diagnosis* / Q000235:genetics*; D004452:Echocardiography; D004562:Electrocardiography; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012720:Severity of Illness Index; D012737:Sex Factors; D055815:Young Adult